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Scleromyxedema: A Case Series Highlighting Long-Term Outcomes of Treatment With Intravenous Immunoglobulin (IVIG).
Medicine (Baltimore). 2008 Jan;87(1):10-20.
Blum M, Wigley FM, Hummers LK.
From
Department of Medicine (MB, FMW, LKH), Division of Rheumatology (FMW,
LKH), Johns Hopkins University School of Medicine, Baltimore, Maryland.
Scleromyxedema
is a rare disease characterized by mucin deposition in skin and other
organs and the production of a monoclonal IgG protein. Herein we
describe our experience with a series of patients with this condition
and specifically focus on the use of intravenous immunoglobulin (IVIG)
for long-term management. We retrospectively reviewed the clinical
manifestations of 10 patients evaluated at our center, highlighting the
potential organ involvement.
We found that systemic manifestations of
the disease are common and often mimic those seen in systemic
sclerosis. Eight of the 10 patients were treated with IVIG with a 100%
complete or partial response rate. Treatment was initiated at a dose of
2 g/kg (total dose, divided over 2-5 days) for 6 months of initial
therapy. In each case where IVIG was used, maintenance infusions are
required to preserve disease control. We highlight the long-term use of
this medication in several cases. We discuss the potential therapeutic
benefit of IVIG in this condition, where the pathophysiology of the
disease is poorly understood, and underscore new data on the potential
mechanism of action of IVIG therapy.
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